Evans Syndrome After Successful Immunosuppressant-Free Living-Donor Liver Transplant.

Evans syndrome is an uncommon disease characterized by a combination of autoimmune hemolytic anemia and autoimmune thrombocytopenia concomitantly or sequentially with a positive direct Coombs test in the absence of any underlying known cause. Here, we present a case of an adult patient who underwent living-donor liver transplant that was preceded by bone marrow transplant 20 years earlier from the same HLA identical donor and who received a single-agent immunosuppressive therapy for only 2 months as prophylaxis against graft-versus-host disease. Two months after transplant, he developed Evans syndrome with severe anemia and thrombocytopenia. After administration of steroids and intravenous immunoglobulin, the patient's anemia and thrombocytopenia improved dramatically. Through the 7 years of follow-up, the patient has not developed graft-versus-host disease or acute or chronic rejection. This case demonstrates a rare complication posttransplant and the possibility of functional tolerance of liver grafts after a combined liver and bone marrow transplant from the same donor.